[Adults with congenital heart disease: treatment and medical problems]. / Erwachsene mit angeborenen Herzfehlern : Versorgungssituation und medizinische Probleme.

By improvement of the medical care, children with congenital heart disease survive in much greater numbers: the figure of ACHD-patients in Germany is estimated up to 203,000 to 301,000. The need for a specialized care of these patients is accepted by the scientific societies, who introduced a certification for doctors and centers and created guidelines. The medical problems include imaging, treatment of arrhythmia, congestive heart failure and cyanosis, prophylaxis of infectious endocarditis, exercise and pregnancy. For the most frequent defects, indication for treatment and postoperative care is summarized.

Balloon dilation of native aortic coarctation in infancy.

Balloon dilation of aortic coarctation in neonates and infants remains controversial. Between 2/1985 and 8/1999, 80 patients <12 months of age, with native aortic coarctation underwent balloon dilation. The systolic pressure gradient across the stenosed area was reduced significantly acutely from a mean of 45.6+/-19.4 mmHg to 17.9+/-13.8 mmHg. In 55 (68.8%) patients, the procedure was initially successful with a residual gradient of <20 mmHg. In 12.5% of patients, intimal tears were detected after dilation. In 21.3% of patients, obstruction of the femoral artery occurred, which responded to heparin or streptokinase in all. Two patients developed aneuryms immediately after the first intervention. In 1 case, surgery was performed with a successful aneurysmectomy. Severe complications or death in relation to the procedure were not detected. Long-term follow-up was obtained in 66 of 80 (82.5%) patients in a period between 6 to 174 months (median 29 months). In 22/66 (33%) of the infants, within a mean period of 10.9+/-15.2 months after first intervention, a redilation was necessary. Sixteen of 22 were successful procedures. In the remaining six patients, surgery was performed electively. A total of 30/66 who had follow-up (45%) patients remained free from reintervention or surgery after the first procedure. Further analysis of the data according to age showed that neonates and infants < or =3 months of age had a 90% and 62% higher residual stenosis rate, respectively, than infants >3 months. In this young age group, balloon dilation can only be recommended as palliation in young infants with severe left venticular dysfunction or in the case surgery is prohibitive for other reasons.

Effective doses to patients from paediatric cardiac catheterization.

The Council Directive of the European Communities 97/43/Euratom requires dose assessment, especially for X-ray examinations of children and if high doses to the patient are involved. Both these aspects apply in cardiac catheterization and angiocardiography of children. Effective doses are a good indicator of radiation risk, particularly for leukaemia. Effective doses have been determined for 2114 infants and children undergoing cardiac catheterization from 1984 to 1996 at the University Hospital in Essen. Conversion factors (effective dose/dose-area product) were calculated based on direct dose-area product measurements for posteroanterior (PA) and lateral (Lat) projections as well as on patient records and examination details. The factors are calculated for eight age groups of children, taking into account the X-ray tube voltage for fluoroscopy and cine-film sequences, with and without zoom mode. Frequency distributions are presented for 2114 patients, for dose-area product, number of angiographic examinations (each combined with one cine-film sequence both PA and Lat) and for calculated effective doses. Highest effective doses are found in newborns (18.0 mSv and 6.5 mSv 90th and 50th percentiles, respectively) compared with adolescents of 15-21 years (8.0 mSv and 3.0 mSv 90th and 50th percentiles, respectively). Effective dose for cardiac catheterization is highest for newborns, in spite of lowest measured dose-area products, because the decreased value of the conversion factors overcompensates for the increase of dose-area product with age. This is especially important because of the higher tumour risk for equal effective dose for young children compared with adults.

Closure of the zone of apposition at correction of complete atrioventricular septal defect improves outcome.

OBJECTIVE: Outcome after correction of atrioventricular septal defect depends to a great deal on the postoperative function of the left atrioventricular valve. The related role of the zone of apposition ('cleft') has been debated: should it be closed (bileaflet repair) or should it be left untouched (trileaflet repair)? This study aims to answer the question by comparing the outcome of patients treated according to these two approaches. METHODS: We reviewed all our patients who underwent repair of complete atrioventricular septal defect from 1984 to 1997 and selected those in whom the closure of the zone of apposition in principle would have been possible. Two groups with similar characteristics were constituted: group I (n=63), where the zone of apposition was deliberately not closed as part of a trileaflet repair (postoperative open zone of apposition) and group II (n=96), where it was electively closed as part of a bileaflet AV valve repair (closed zone of apposition). Since we changed from a trileaflet to a bileaflet repair in 1987, the two groups differ in terms of size and length of follow-up. Outcome was compared with regard to survival and freedom from reoperation for left atrioventricular valve incompetence. Late atrioventricular valve function was evaluated by Echo-Doppler. For statistical analysis, we used Chi-square or Fisher's exact test, the Mann-Whitney test and the log-rank test for comparison of Kaplan-Meier curves. The difference was considered statistically significant with a P-value of 0.05 or less. RESULTS: Early mortality was 9.5% (6/63) in group I and 3.1% (3/96) in group II (P=0.16). Actuarial survival after 1, 4 and 8 years was 80.4, 68.4 and 64.8%, respectively, for group I. Actuarial survival for group II was 94.7, 92.1 and 92.1% (P=0.0002). Freedom from reoperation for left atrioventricular valve regurgitation was 90.2, 85.6 and 77.8% for group I at the same time interval. It was a constant 97.9% for group II (P=0.0016). At reoperation, left atrioventricular valve regurgitation was present through the open zone of apposition in 63% of group I cases. The follow-up is 96% (126/131) complete. An increase in degree of left atrioventricular valve incompetence was noted in 28% (11/39) of group I cases and in 9% (8/87) of group II cases (P=0.0131). CONCLUSION: This study demonstrates the advantage of closing the zone of apposition ('cleft') as part of repair of complete atrioventricular septal defect. Survival, freedom from reoperation for left atrioventricular valve incompetence and over-all outcome were more favourable in patients of group II. The zone of apposition should be surgically addressed whenever the morphology of the left atrioventricular valve allows for closure without producing stenosis.

Double-chambered right ventricle in 73 patients: spectrum of the disease and surgical results of transatrial repair.

OBJECTIVE: To review the spectrum of double-chambered right ventricle (DCRV) and the outcome of surgical repair in patients diagnosed between February 1988 and March 1999. DESIGN: The charts of patients with DCRV were studied. SETTING: Tertiary care hospital. PATIENTS AND METHODS: A total of 73 patients were identified. Sixty-nine underwent surgical repair, while four are awaiting surgery. The repair was through a transatrial approach in 61 patients, while in eight an additional ventriculotomy was performed. MAIN RESULTS: An associated ventricular septal defect (VSD) was present in 56 of 73 patients (77%). These patients were significantly younger (P<0.05) than the 17 patients without a VSD. Among patients with a VSD, the 31 requiring patch closure were significantly younger than the 25 patients having direct closure. Five older patients among those with intact septum had impaired right ventricular (RV) function as well as higher intraventricular gradients. At surgery the intraventricular obstruction was relieved by myomectomy. There was no hospital or late mortality. Following surgery, at a mean follow-up of 13.6 months, no increase in the intraventricular gradient was detected by Doppler echocardiography. CONCLUSIONS: The development of DCRV is associated with VSD in early life. The probability of the presence of a VSD decreases with age. The disease is progressive, resulting in increased intracavitary gradient within the RV and in RV impairment if it is not treated in a timely fashion. Transatrial repair is safe with excellent midterm results. In the presence of high gradients within the RV, a ventriculotomy may be necessary to obtain acceptable results.

[Interventions in congenital heart disease and their sequelae in adults]. / Neue Therapiemöglichkeiten in der interventionellen Kardiologie. Bedeutung für angeborene Herzfehler im Erwachsenenalter.

The advancements of cardiac surgery over the last decades led to larger numbers of patients with operated congenital heart diseases surviving into adulthood. In Germany it is estimated that over 120,000 adults have operated congenital heart diseases. Five to 7% of them will need yearly hospital admissions. Interventional procedures are additional tools used to treat these patients with various sequelae or residua (Table 1). In the following review we concentrate on 2 different interventional procedures: dilatation and stent implantations for treatment of stenosis and the different devices used for the closure of shunt lesions. For congenital valvular pulmonary stenosis, balloon dilatation is the therapy of choice regardless the age of the patient. Stent implantation for the treatment of peripheral pulmonary stenosis (e.g., after previous systemic pulmonary shunts) can decrease the need for redo surgery, which is accompanied with increased risk. Stent implantations proved also to be useful to treat stenoses after Mustard patch in patients with transposition of the great arteries, after Fontan procedures or dealing with the rare pulmonary venous stenosis. In contrast, dilatation of bioprosthesis and conduit stenosis are not promising. Balloon dilatation of valvular aortic stenosis is an accepted therapy in childhood up to adolescents. Table 2 compares a surgical series including many infants with critical aortic stenosis with a series of balloon dilatation in children and another one in adults regarding lethality, complications, and results. Table 3 illustrates the immediate and late results of balloon dilatation of aortic coarctation in 3 different studies. The high recurrence rate in infants made clinicians refrain from taking this age group for balloon dilatation. In children and adult patients, good results are reported (75% reduction of gradients). The complication rate is low (2.3 to 3.3%) and aneurysm formation rate seldom (1 to 7%). Stenosed aorto-pulmonary collaterals will rarely need balloon dilatation. Surgical closure of atrial septal defect is a low risk procedure with a very low rate of residual shunts (2%). Of the 5 available devices for transcatheter closure of atrial septal defect Type II, only 2 occluders are in use in Germany, the Clamshell and the Amplatzer device. The largest clinical studies of the different systems, their efficacy, complications and residual shunt rate are presented in Table 4. For the deployment of these occluders a TEE is always needed. There are many more systems in clinical use to close the patent arterial duct (PDA) (Table 5). The Ivalon plug as well as the Rashkind device have probably only historical value. Different types of coils (Gianturco, Cook detachable, PFM) are now in use worldwide. The reason for their widespread use, besides their easy application, is the fact that most coils are relatively cheap and need only small sheaths for deployment. Their further evaluation identified a residual shunt rate of 5% as well as a number of complications (embolization, hemolysis, stenosis of the left pulmonary artery) in 0 to 6%. For the large PDA the Amplatzer device has recently been introduced. An additional indication for the use of the different occluding devices are aorto-pulmonary collaterals, venovenous fistulae, pulmonary or coronary artery fistulae. Aorto-pulmonary collaterals are often associated with complex cardiac lesions and occasionally appear after palliative procedures. An excellent cooperation between adult and pediatric cardiologists is needed in order to offer the group of adults with congenital heart diseases an adequate and comprehensive management.

Immunosuppressive therapy of chronic myocarditis in children: three cases and the design of a randomized prospective trial of therapy.

Three infants, each with a clinical picture of dilated cardiomyopathy, underwent endomyocardial biopsy. Immunohistologic analysis revealed chronic myocarditis. In one infant, a postviral etiology of chronic myocarditis could be assessed on the basis of molecular techniques. Therapy with azathioprine and prednisone resulted in the normalization of echocardiographic findings. Based on these observations, a randomized, multicenter treatment study of chronic myocarditis in children (TCMC) has been initiated.

[Change in right ventricular diastolic function in children and adolescents with mucoviscidosis--a Doppler echocardiographic study]. / Rechtsventrikuläre diastolische Funktionsänderung bei Kindern und Jugendlichen mit Mukoviszidose--eine dopplerechokardiographische Studie.

The present study investigated the right ventricular filling pattern with Dopplerechocardiography in childhood and adolescent patients (n = 31) with cystic fibrosis and in normal subjects. The results show that, as the pulmonary pressure increases (RPEP/AT > or = 1.0), the peak flow velocity during atrial contraction and the triangle under the atrial velocity are significantly increased while the acceleration half-time is significantly decreased. These are sensitive parameters for right ventricular diastolic dysfunction.

[Transcatheter obliteration of patent ductus arteriosus in young adults with the Rashkind occluder]. / Transkatheter-Verschulss des offenen Ductus arteriosus bei jungen Erwachsenen mit dem Rashkind-Occluder.

Fourteen young adult patients between 14 and 29 years of age underwent 15 procedures of transcatheter occlusion of patent ductus arteriosus. In all patients but one, the attempt to implant the device was successful (92%). In one patient the ductus was judged to be too large for occlusion and the patient was sent for surgery. In no patient did the device embolize. A total of 6/13 (46%) patients was discharged home with no residual shunt. Follow-up data (between 3 and 18 months) are available in 10/13 patients. To date, 9/13 (70%) had total occlusion of their duct, in one patient after reocclusion.

[Myocarditis in childhood]. / Die Myokarditis im Kindesalter.

Infectious heart diseases in childhood are--with less than 1% of hospital admissions--rare, but serious diseases. Among several causes of myocarditis in our region virus myocarditis plays the most important role. Besides of the acute course an autoimmune mediated chronic myocarditis and the transition to dilated cardiomyopathy are observed. The patho-histological assessment depends also on subjective influences. Clinical diagnosis is based on cardiac symptoms (Adams-Stokes, congestive heart failure, LV-dilatation), ECG-changes with increased enzyme levels and positive virus-serology or endomyocardial biopsy. Immunoserology and -histology as well as in-situ-hybridization can support diagnosis, echocardiography and eventually heart catheterization exclude other causes. In respect to therapy, ACE-inhibitors are a substantial improvement of conventional therapy of heart failure, while immunosuppressive therapy of chronic myocarditis has to assessed.

[Sudden death caused by a heart tumor]. / Plötzlicher Tod infolge Herztumors.

Primary cardiac tumors in infancy and childhood are extremely rare. In the young age group the clinical symptoms often are variable and can make diagnosis difficult. Depending on the histological character and especially the localisation of the mass the clinical appearance may lead to the first diagnosis or at least to further investigation. While myocardial tumors (rhabdomyomas and fibromas) mainly cause rhythmic disturbances, the intracavitary tumors (like myxomas) frequently disturb hemodynamics. Echo-cardiography is the main diagnostic technique, followed by angiocardiography, computertomography and nuclearmagneticresonance imaging.

Endomyocardial biopsy in infants and children: experience in 60 patients.

In 60 children, aged between 1 month and 22 years (median 3.54 years) and with a body weight of 3-67 kg (median 12.6 kg), transvascular endomyocardial biopsy (EMB) was performed from the right (35 children) or left ventricle (30 children). The specimens were investigated by light and electron microscopy. There were three indications for biopsy: (1) poorly functioning, dilated left ventricle (seven patients with endocardial fibroelastosis, 16 with dilated cardiomyopathy, six from healing/healed or chronic myocarditis); (2) unexplained left ventricular hypertrophy (10 with hypertrophic cardiomyopathy, four with secondary hypertrophy, three with storage diseases); (3) to answer certain questions in eight children (four with hypoxic and two with cytoxic myocardial damage). Retrospectively, there were five nonindicated biopsies. There were no serious complications. Biopsies were diagnostic in 11.7% of cases, helpful in 71.7%, and of no help in 16.6%. Thus even in childhood endomyocardial biopsy is a diagnostic tool which can add useful information on the etiology or pathogenesis of an underlying myocardial disease.

[Interventional cardiology in children]. / Interventionskardiologie im Kindesalter.

Interventional heart catheterizations are catheterizations for a therapeutic purpose. The widest spread example is the balloon atrioseptostomy (BAS) according to Rashkind, which is performed in patients with transposition of the great arteries preceding interatrial corrective surgery, in patients with right-sided valve atresias and interatrial obstruction and in patients with complex mitral atresia. In 248 BAS, performed within 20 years we observed 26 (= 10.5%) minor and 3 (= 1.2%) lethal complications. When the BAS is ineffective or the atrial septum very muscular, the Park-blade-septostomy may be performed, provided corrective surgery is out of question. intravasal foreign bodies, e.g. catheter fragments can be extracted without harm and risk by the Dotter retriever. This prevents grave complications. Arteriovenous aneurysms, coronary fistulas or aortopulmonary collaterals can be embolized by steel coils, detachable balloons or Ivalon particles. With increasing frequency patent ducts, seldom also atrial or ventricular septal defects are closed by means of the Rashkind occluder. Balloonvalvuloplasty (BVP) of pulmonary or aortic stenosis became generally accepted. In cooperative studies of the German Society of Pediatric Cardiology in 305 BVP's of pulmonary stenosis 4% complications with one late death have been observed. In aortic stenosis of children early mortality was 1.6%, complication rate 20%, in the critical aortis stenosis of infants early mortality was 19%, complication rate 50%. So BVP of pulmonary and non-critical aortic stenosis may be considered as the treatment of choice, while BVP of critical aortic stenosis and other stenosis of valves or vessels has to be further evaluated.

[Aneurysm of the vein of Galen as a possible cause of congestive heart failure: a report of 5 new cases]. / Aneurysma der Vena Galeni als mögliche Ursache für eine kongestive Herzinsuffizienz: Bericht über 5 neue Fälle.

We report on five newborns with an arterio-venous malformation of the vein of Galen. All newborns were cyanotic and in congestive heart failure without any evidence of congenital heart disease. Congestive heart failure in these cases was mainly due to an almost two-fold increase in cardiac output of approximately 8 l/min/m2 (normal: 4.5 l/min/m2). According to previous reports, mortality is very high in patients with this malformation when becoming symptomatic during infancy, and therapy by surgery or embolization is only successful in 10-30%. While three of our patients died shortly after diagnosis because of untreatable heart failure, the other two were operated on either by subtotal ligation of the draining vein or by ligation of 4 arterial feeders. In the first case secondary thrombosis of the aneurysm occurred and cardiac failure subsided. In the second case a large shunt remained and a balloon-embolization was performed successfully. However, in both patients severe neurologic defects occurred, the severity of which remains to be assessed later since the post-operative observation period is only 2 and 5 months, respectively.

Balloon valvuloplasty of pulmonary stenosis in infants and children--co-operative study of the German Society of Pediatric Cardiology.

Balloon valvuloplasty (BVP) of pulmonary stenosis (PST) was performed in 305 infants and children in 20 heart centres of the Federal Republic of Germany. 273 patients suffered from isolated PST. Their age ranged from 3 days to 18 years (mean = 5.85 +/- 4.82 years), their weight from 2.9 to 60 kg. The pressure gradient was measured invasively in the sedated or anaesthetized child, partly by CW Doppler. BVP was performed by mono- or trefoil catheters, mostly oversized. As a result, mean pressure gradient fell from 71.7 +/- 32 mmHg before to 31.8 +/- 25 mmHg immediately after BVP (P less than 0.0001). The gradient was reduced by less than 30% in 13% of the patients, by 30-49% in 23%, and by greater than or equal to 50% in 64%. Among 33 patients with a gradient reduction of less than 30%, nine were aged less than 1 year, nine had dysplastic valves, and nine had a short-term infundibular reaction and good late results. Gradient reduction was not improved with oversized balloons and was independent of the initial gradient. Follow-up for a mean 11.2 +/- 8 months showed a further slight improvement, probably due to regression of infundibular hypertrophy. In the whole group we observed infundibular reaction in 12%, serious complications with one late death in 4%. From the start, BVP proved to be a secure and effective tool for elimination of pulmonary stenosis.

[Secondary immunopathogenesis in myocarditis, diseases of the heart muscles and infarct-associated arrhythmia in childhood and adulthood]. / Sekundäre Immunopathogenese bei Myokarditis, Herzmuskelerkrankungen und infarktassoziierten Rhythmusstörungen im Kindes- und Erwachsenenalter.

In histologically proven or clinically diagnosed perimyocarditis in children and adults alterations in cellular and humoral effector mechanisms are demonstrable: OKIaI-positive B- or activated T-lymphocytes are increased in the peripheral blood, whereas natural killer cell activity is reduced. Antibodies are directed to the membranes of isolated human atrial myocytes and, to a lesser extent with lesser specificity, to endothelial cells and to the extracellular matrix. AMLAs are of diagnostic relevance if they belong to the IgM class, indicating a recent humoral immune stimulation and, if they fix complement, indicating a functional property which is complement associated lysis of target cells. Immunohistological studies demonstrate fixation of immunoglobulins to the autologous biopsy specimens, which are diagnostic only if they also belong to the IgM class and fix complement. In acute rhythm disturbances in the context of a recent "common cold", similar humoral immune reactions can be found in children, which are compatible with a secondary immunopathogenesis after viral illness involving the myocardium.

[Secondary diseases of the heart muscle and their differential diagnosis in childhood]. / Sekundäre Herzmuskelerkrankungen und ihre Differentialdiagnose im Kindesalter.

Possible causes of specific cardiac muscle disease, diagnosis, follow-up and the therapeutic management are discussed on the basis of a series of cases. 8 out of 30 patients who showed the clinical picture of dilative cardiomyopathy (DCM) were found to have a specific cardiac muscle disease. 4 patients had DCM following adriamycin therapy. Fibromuscular dysplasia with renal hypertension, thalassaemia major with secondary haemosiderosis, long-overlooked and untreated athyroidism each caused one case of dilative specific cardiac muscle disease. Once DCM was preceded by the Kawasaki syndrome for over 2 years. Amongst 47 patients with hypertrophic cardiomyopathy there were two children who had undergone ACTH treatment, 6 children born of diabetic mothers, 4 cases of Pompe's disease, and one patient with hypothyroidism resulting in reversible hypertrophy of the cardiac muscle. Different neurodegenerative diseases were associated with cardiac muscle disease in 4 cases, partly dictating the clinical course. Extremely rare was the development over 6 years of cardiac hypertrophy following a burns injury.

[Endomyocardial biopsy in childhood--experiences in 60 pediatric patients]. / Endomyokardbiopsie im Kindesalter--Erfahrungen bei 60 pädiatrischen Patienten.

In 60 pediatric patients, aged from 1 month to 22 years (median 3.54) and a bodyweight of 3 to 67 kg (median 12.6 kg) transvascular endomyocardial biopsy was performed from the right (35 patients) or left ventricle (30 patients). The specimens were investigated by light and electron microscopy. Immune serological investigations were performed in 22 patients, immune histological examinations in eight. There were three indications for biopsy: a. 29 children had a poorly functioning, dilated left ventricle. Of these, seven suffered from endocardial fibroelastosis, 16 from dilated cardiomyopathy, six (plus one control-biopsy) from healing/healed or chronic myocarditis. b. 17 children showed inadequate left ventricular hypertrophy. Of these, ten suffered from HCM, four from secondary hypertrophy, three from storage diseases. c. Various questions were answered in eight children - four with hypoxic, two with cytotoxic myocardial damage. There were five misindications, retrospectively. We observed no serious complications. Evaluation of biopsy revealed diagnostic findings in 11.7%, was helpful in 71.7% and of no help in 16.6%. Hence, even in childhood, endomyocardial biopsy is a diagnostic tool which can contribute useful information on the etiology or pathogenesis of the underlying myocardial disease.

Dilated cardiomyopathy in childhood: problems of diagnosis and long-term follow-up.

Clinical profile and course of 13 infants and children (median age 2 years, range 2 months to 17 years) with dilated cardiomyopathy are presented. Endocardial fibroelastosis and myocarditis were excluded by transvascular endomyocardial biopsy. Elevated cardio-thoracic ratio (mean 0.67), depressed fractional shortening (mean 13%) and cardiac index (mean 2.52 l min-1 m-2) were the indicators of congestive heart failure. During a mean follow-up of 41 months (range 6 to 204 months) five patients died, four remained in a stable condition, four improved. The main complications were rhythm disturbances (23%) and thrombus formation (15%). We could not identify any predictors of survival.

[Pulmonary artery loop and dysplastic tracheal stenosis. Case report with pre- and postoperative spirometry and discussion of the value of diagnostic signs]. / Pulmonalarterienschlinge und dysplastische Trachealstenose. Fallbericht mit prä- und postoperativer Spirometrie und Diskussion der Wertigkeit der diagnostischen Zeichen.

Since his second week of life a boy, with pulmonary artery sling now 8 3/4 years old suffered from mainly expiratory stridor during exercise and dyspnoe during bronchopulmonary infections, twice in a life-threatening manner. The indirect diagnostic signs were all negative, bronchoscopy revealed a narrow distal trachea and a pulsating stenosis of high degree at the bifurcation. Bronchographic findings were characteristic. Angiocardiographically the retrotracheal run of the left pulmonary artery could only be proved by marking the trachea with a catheter. After operation at the age of 8 1/4 years, the boy had no relevant subjective complaints during a follow-up of 6 months. Spirometric lung function tests before and after corrective operation revealed a considerable obstruction of central intrathoracic airways that persisted after operation, only peak flow improved significantly. According to the bronchological findings the reason therefore is a dysplastic tracheobronchial stenosis, which is seen often in combination with PAS.